Presence Of Ventricular Septal Defect Research Articles

Progressive changes in residual gradient after aortic coarctation repair and its role in the prediction of reintervention: A longitudinal data analysis.

Repair of aortic coarctation through left thoracotomy is the standard treatment when anatomically feasible. Long-term outcomes are well studied, including the need for reintervention. However, the timely variation in residual gradients across the repaired segment is ill-defined. The aim of this work was to study the progressive changes of estimated peak gradient (ePG) acquired by transthoracic continuous-wave Doppler echocardiography across the aortic arch after repair and to assess the role of timing of assessment and values of ePG in prediction of reintervention. All eligible patients for this study who underwent aortic coarctation repair through left thoracotomy from 2001 to 2017 were reviewed. Details of the aortic arch dimensions and associated lesions were obtained by transthoracic echocardiography (TTE). The primary outcome was the ePG across the aortic arch after repair. Longitudinal data analyses with mixed effect modeling were used to determine independent predictors for ePGs. A total of 312 patients were included. Median age and weight were 30 days and 4 kg, respectively. Associated lesions included ventricular septal defect (VSD) (53%), bicuspid aortic valve (53%) and mitral stenosis (25%). Over 15-years follow-up the freedom from reintervention was 92.3%, while 24 out of the 312 patients underwent reintervention (7.7%). Longitudinal data analyses of serial 2566 TTE studies were done. The graphical display showed that the ePG across coarctation area in the first postoperative TTE was the most notable difference between those who underwent reintervention and those who did not. Further testing with proportional hazard and logistic regression modeling confirmed this finding. The area under receiver operating curve statistics showed that an ePG of 25 mmHg is an optimal cutoff value for the prediction of the reintervention. The ePG acquired in the first postoperative TTE is the most important predictor for reinterventions. The presence of VSD is associated with decreased ePGs. We propose that an ePG in the first postoperative TTE of 25 mmHg or more is a strong predictor for the need of reintervention.

Aortic Atresia or Complex Left Outflow Tract Obstruction in the Presence of a Ventricular Septal Defect.

Severe left outflow tract obstruction (LVOTO) is not always associated with hypoplastic left heart syndrome (HLHS). Aortic valvar atresia or complex LVOTO in the presence of a large ventricular septal defect (VSD) are a rare group of lesions that offer the possibility of biventricular repair. The Yasui procedure is the commonest surgical approach which can be performed as a primary neonatal correction or as a staged procedure with a Norwood followed by a subsequent Rastelli. This article reviews the surgical outcomes and decision-making process. Both strategies are reasonable with the trend toward staged procedure in the setting of the additional interrupted arch, with neonatal survival of > 90% in the modern era and excellent long-term survival. Re-intervention is mostly related to conduit revision and the need for re-operation for LVOTO is rare. Deciding between conventional repair and the Yasui in cases of LVOTO/VSD can be difficult and there are no uniform accepted criteria. In a typical neonate, an aortic annulus < 4.5 mm is generally the limit of acceptability for a conventional repair. In selected cases of LVOTO/VSD, an alternative to the Yasui is the Ross-Konno. Retrospective comparisons between the 2 techniques are difficult due to differing patient characteristics (especially associated with mitral valve disease) but the neonatal Ross has been associated with higher early mortality.

Clinical Presentation of Preterm Infants with Ventricular Septal Defect.

Ventricular septal defects (VSDs) are the most common congenital heart diseases; however, case reports of preterm infants with VSD are limited. The aim of this study is to share our experience with preterm infants with VSD and to record their short-term outcomes. Between January 2000 and December 2017, 32 preterm infants with VSD were admitted to our neonatal intensive care unit at gestational age < 32 weeks. Of these, 9 were excluded by exclusion criteria. The size and location of the VSD, details of treatment, and neonatal prognosis were retrospectively reviewed from the medical records. Among the 23 preterm infants, the median gestational age was 29.4 weeks (25.0-31.3 weeks) and the median birthweight was 924 g (524-1,526 g). There were 9 infants with VSD < 2 mm and 14 infants with VSD ≥ 2 mm. For the 9 infants with VSD < 2 mm, no medical or surgical treatments for VSDs were undertaken. Of the 14 infants with VSD ≥ 2 mm, 8 (57.1%) underwent medical and surgical treatment. Surgical treatment was performed more frequently in infants with VSD ≥ 2 mm than in those with VSD < 2 mm (P = 0.007). In preterm infants, the presence of VSD ≥ 2 mm increases the risk of surgical interventions and significant patent ductus arteriosus. It is important to encourage treatment for preterm infants with VSD ≥ 2 mm, including surgical interventions, in cooperation with pediatric cardiologists.

Is analysis of physiological late gestation ventricular-arterial disproportion futile?

Ventricular-arterial disproportion is a frequent observation in late gestation and mostly considered physiological. It is also an indirect prenatal sign for postnatal coarctation. To investigate the remnant risk for coarctation and its possible risk factors in a fetal population with physiological late gestation asymmetry. This was a monocentric, retrospective study conducted from 2014 till 2017 in a Fetal Cardiology Unit. All foetuses referred for third trimester asymmetry with pulmonary artery/aorta ratio > 1.2 were included. Annular sizes, arterial Z-scores, valve ratios, isthmus size if available and minor associated cardiac defects were analyzed. One hundred and one foetuses were referred at a mean gestational age of 33.5 SA. After foetal echocardiography, 2% (3/151) were considered at high risk for coarctation because of a hypoplastic arch and/or a small left outflow tract or isthmus. They were scheduled to deliver in a tertiary referral center. Two of these three newborns developed coarctation. In 98% of foetuses, asymmetry was considered physiological with a normally sized aortic arch without the “shelf sign”. All infants had an early ambulatory echocardiography that revealed coarctation in 7/148 cases (4.7%). There were no significant differences in mean arterial annular sizes, aortic and pulmonary Z-scores, pulmonary artery/aorta ratio, atrioventricular annular sizes, tricuspid/mitral valve ratio, presence of ventricular septal defect or left persisting superior caval vein between the two groups. Remnant risk for coarctation in a foetal population with physiological late gestation asymmetry is < 5%. Prenatal counselling can be reassuring since the majority of infants will have a normal heart. However early cardiac evaluation of these newborns remains recommended to exclude the rare event of postnatal coarctation in absence of prenatal predictive signs.

Is analysis of physiological late gestation ventricular-arterial disproportion futile?

Ventricular-arterial disproportion is a frequent observation in the third trimester and mostly considered physiological. It is also an indirect prenatal sign for postnatal coarctation. We sought to investigate the remnant risk for coarctation and its possible risk factors in a fetal population with physiological late gestation asymmetry. This was a monocentric, retrospective study conducted from 2014 till 2017 in a Fetal Cardiology Unit. All fetuses referred for third trimester asymmetry with pulmonary artery/aorta ratio > 1.2 were included. Annular sizes, arterial Z-scores, valve ratios, isthmus size if available and minor associated cardiac defects were analyzed. 151 fetuses were referred at a mean gestational age of 33.5 SA. After fetal echocardiography, 2% (3/151) were considered at high risk for coarctation because of a hypoplastic arch and/or a small left outflow tract or isthmus. They were scheduled to deliver in a tertiary referral center. Two of these 3 newborns developed coarctation. In 98% of fetuses asymmetry was considered physiological with a normally sized aortic arch without the “shelf sign”. All infants had an early ambulatory echocardiography that revealed coarctation in 7/148 cases (4.7%). There were no significant differences in mean arterial annular sizes, aortic and pulmonary Z-scores, pulmonary artery/aorta ratio, atrioventricular annular sizes, tricuspid/mitral valve ratio, presence of ventricular septal defect or left persisting superior caval vein between the two groups. Remnant risk for coarctation in a fetal population with physiological late gestation asymmetry is < 5%. Prenatal counselling can be reassuring since the majority of infants will have a normal heart. However early cardiac evaluation of these newborns remains recommended to exclude the rare event of postnatal coarctation in absence of prenatal predictive signs.

A rare association of congenital mid-muscular ventricular septal defect with acute anterior wall myocardial infarction in the elderly: Clinical and hemodynamic implications

We report a rare association of acute myocardial infarction (AMI) in an elderly patient who had undiagnosed mid-muscular ventricular septal defect (VSD). The patient underwent primary percutaneous coronary angioplasty of the left anterior descending artery. It is uncommon to survive with VSD at this age, and the association of it with AMI is still rare. Whether the presence of congenital heart disease poses them for the increased risk of coronary artery disease is a matter of further investigation. Furthermore, the presence of VSD while they incur myocardial infarction affects the prognosis of the patient is not clear.

Perinatal Natural History of the Ts1Cje Mouse Model of Down Syndrome: Growth Restriction, Early Mortality, Heart Defects, and Delayed Development.

BackgroundThe Ts1Cje model of Down syndrome is of particular interest for perinatal studies because affected males are fertile. This permits affected pups to be carried in wild-type females, which is similar to human pregnancies. Here we describe the early natural history and growth profiles of Ts1Cje embryos and neonates and determine if heart defects are present in this strain.MethodsPups were studied either on embryonic (E) day 15.5, or from postnatal (P) day 3 through weaning on P21. PCR amplification targeting the neomycin cassette (present in Ts1Cje) and Sry (present in males) was used to analyze pup genotypes and sex ratios. Body weights and lengths, as well as developmental milestones, were recorded in Ts1Cje mice and compared to their wild-type (WT) littermates. Histological evaluations were performed at E15.5 to investigate the presence or absence of heart defects. Pups were divided into two groups: Ts1Cje-I pups survived past weaning and Ts1Cje-II pups died at some point before P21.ResultsTs1Cje mouse embryos showed expected Mendelian ratios (45.8%, n = 66 for Ts1Cje embryos; 54.2%, n = 78 for WT embryos). Histological analysis revealed the presence of ventricular septal defects (VSDs) in 21% of Ts1Cje E15.5 embryos. After weaning, only 28.2% of pups were Ts1Cje (185 Ts1Cje out of 656 total pups generated), with males predominating (male:female ratio of 1.4:1). Among the recovered dead pups (n = 207), Ts1Cje (63.3%, n = 131, p<0.01) genotype was found significantly more often than WT (36.7%, n = 76). Retrospective analysis of Ts1Cje-II (pre-weaning deceased) pups showed that they were growth restricted compared to Ts1Cje-I pups (post-weaning survivors). Growth restriction correlated with statistically significant delays in achieving several neonatal milestones between P3 and P21 compared to Ts1Cje-I (post-weaning survivors) neonates and WT littermates.ConclusionsTs1Cje genotype is not associated with increased early in utero mortality. Cardiac defects, specifically VSDs, are part of the phenotype in this strain. There is increased neonatal mortality in Ts1Cje pups, with sex differences observed. Ts1Cje mice that died in the neonatal period were more likely to be growth restricted and delayed in achieving neonatal developmental milestones.

Prenatal Diagnosis of Interrupted Aortic Arch: Usefulness of Three-Vessel and Four-Chamber Views.

Interrupted aortic arch (IAA) is fatal if not diagnosed. Prenatal diagnosis is helpful, but it is difficult to detect IAA and even more so to differentiate types A and B prenatally. Our objectives were to find a way to detect IAA using 2 views-three-vessel view (3VV) and four-chamber view (4CV)-and to differentiate between types A and B. We retrospectively analyzed fetal echocardiographic images and medical records of eight IAA patients. All eight patients had a ventricular septal defect (VSD) on 4CV. The aorta/main pulmonary artery (Ao/MPA) diameter ratio on 3VV was significantly low, which is characteristic of type B IAA. The left/right ventricular diameter (LV/RV) ratio on 4CV was 0.61± 0.17 for type A and almost 1.0 for type B. The thymus was not observed on 3VV in some type B IAA patients. These findings suggest that we could increase the number of prenatal diagnoses of IAA using the Ao/MPA ratio on 3VV and the presence of VSD on 4CV. Additionally, we could differentiate types A and B with the LV/RV ratio on 4CV, the Ao/MPA ratio, and the presence of a thymus on 3VV, which results in better management of IAA after birth.

An analysis of cardiac defects and surgical interventions in 84 cases with full trisomy 18.

Trisomy 18 (Edwards syndrome) is the second most common autosomal trisomy after trisomy 21. Medical issues commonly include cardiac defects, such as ventricular septal defect (VSD) and atrial septal defect (ASD). If untreated, these conditions can contribute to the associated infant mortality. The objective of the study was review parent-reported information on 84 cases with full trisomy 18 focusing on prenatal and postnatal assessment and confirmation of cardiac defects and on subsequent treatment with cardiac surgery and post-surgery outcomes. At birth, 65 parent responses indicated the presence of VSD (77.4%), 38 ASD (45.2%), and 50 patent ductus arteriosus (PDA) (59.5%). The presence of multiple cardiac defects was also analyzed including 25 cases with VSD, ASD, and PDA at birth. The total reduced to 18 at survey completion. Twenty-four cases had one or more cardiac defects repaired for a total of 34 corrective surgeries. Age at surgery varied from 2 weeks to 41 months of age with most performed under 1 year of age. Twenty-one cases were still living at the time of survey completion (87.5%). From these date we provide recommendations and implications.

Short-Term Outcomes of Two Surgical Techniques for the Treatment of Coarctation of the Aorta in Infants: Subclavian Flap Repair and Resection with Extended End-to-End Anastomosis Technique

Background: The aims of the study were to analyze the importance of two different surgical procedures, and to determine outcomes for neonates with coarctation of the aorta in two newly established centers. Methods: Outcomes of two different surgical repairs for coarctation of the aorta in 43 infants were evaluated retrospectively. The study was designed as a nonrandomized, cross-sectional study. The subclavian flap repair was applied to 22 patients (51%) and resection with extended end-to-end anastomosis technique to 21 patients (49%). After all operative survivors were followed up with a mean follow-up of 1.8 ± 0.8 years, data analyzed with t-test and the p value < 0.05 were considered statistically significant. Results: The overall mortality rate was 4.6%. Forty mmHg gradients were determined in a patient from Resection Group postoperatively in the fifteenth month. After the balloon angioplasty, the gradient decreased to 25 mmHg. The presence of ventricular septal defect (p = 0.094) was the only significant predictor of adverse short-term outcome among the associated cardiac defects analyzed. The first-year survival rate was 100% in both groups in isolated coarctation (p = 0.965), however; such rate was found as 100% and 93.25% in Waldhausen Group and Resection Group, respectively in complex coarctation (p = 0.294). Conclusions: Both the subclavian flap repair and resection with extended end-to-end anastomosis for coarctation of the aorta in infants provide excellent short-term outcomes with lower recurrence rates requiring surgery or angioplasty.

A nationwide analysis of clinical outcomes among newborns with esophageal atresia and tracheoesophageal fistulas in the United States

BackgroundThe aim of this study was to examine national outcomes in newborn patients with esophageal atresia and tracheoesophageal fistula (EA/TEF) in the United Sates. MethodsKid's Inpatient Database (KID) is designed to identify, track, and analyze national outcomes for hospitalized children in the United States. Inpatient admissions for pediatric patients with EA/TEF for kid's Inpatient Database years 2000, 2003, 2006, and 2009 were analyzed. Patient demographics, socioeconomic measures, disposition, survival and surgical procedures performed were analyzed using standard statistical methods. ResultsA total of 4168 cases were identified with diagnosis of EA/TEF. The overall in-hospital mortality was 9%. Univariate analysis revealed lower survival in patients with associated acute respiratory distress syndrome, ventricular septal defect (VSD), birth weight (BW) < 1500 g, gestational age (GA), time of operation within 24 h of admission, coexisting renal anomaly, imperforate anus, African American race, and lowest economic status. Multivariate logistic regression identified BW < 1500 g (odds ratio [OR] = 4.5, P < 0.001), operation within 24 h (OR = 6.9, P < 0.001), GA <28 wk (OR = 2.2, P < 0.030), and presence of VSD (OR = 3.8, P < 0.001) as independent predictors of in-hospital mortality. Children's general hospital and children's unit in a general hospital were found to have a lower mortality rate compared with not identified as a children's hospital after excluding immediate transfers (P = 0.008). ConclusionsBW < 1500 g, operation within 24 h, GA < 28 wk, and presence of VSD are the factors that predict higher mortality in EA/TEF population. Despite dealing with more complicated cases, children's general hospital and children's unit in a general hospital were able to achieve a lower mortality rate than not identified as a children's hospital.

Earlier Arterial Switch Operation Improves Outcomes and Reduces Costs for Neonates With Transposition of the Great Arteries

This study sought to examine the impact of surgical timing on major morbidity and hospital reimbursement for late preterm and term infants with dextrotransposition of the great arteries (d-TGA). Neonatal arterial switch operation is the standard of care for d-TGA. Little is known about the effects of age at operation on clinical outcomes or costs for these neonates. We conducted a retrospective cohort study of infants at≥36 weeks' gestation, with d-TGA, with or without ventricular septal defects, admitted to our institution at 5 days of age or younger, between January 1, 2003 and October 1, 2012. Children with other cardiac abnormalities or other major comorbid conditions were excluded. Univariable and multivariable analyses were performed to determine the effects of age at operation on major morbidity and hospital reimbursement. A total of 140 infants met inclusion criteria. Reimbursement data were available for them through January 1, 2012 (n= 128). The mortality rate was 1.4% (n= 2). Twenty percent (n= 28) experienced a major morbidity. The median costs were $60,000, in 2012 dollars (range: $25,000 to $549,000). The median age at operation was 5 days (range: 1 to 12days). For every day later that surgery was performed, beyond day of life 3, the odds of major morbidity increased by 47% (range: 23% to 66%, p< 0.001) and costs increased by 8% (range: 5% to 11%, p<0.001), after considering the effects of sex, birth weight, gestational age, year at which surgery was performed, transfer, weekend admission, insurance, surgeon, septostomy, bypass and cross-clamp times, and the presence of ventricular septal defects or abnormal coronary anatomy. Delay of neonatal arterial switch operation beyond 3 days is significantly associated with increased morbidity and healthcare costs.

Arterial Switch for Transposition With Left Outflow Tract Obstruction: Outcomes and Risk Analysis

The long-term results and indications of the arterial switch operation (ASO) for transposition of the great arteries (TGA) and anatomic left ventricular outflow tract obstruction (LVOTO) remain undetermined. The aims of this study were to determine long-term outcomes and prognostic factors in this specific population. Between 1986 and 2011, 55 patients with TGA and anatomic LVOTO underwent ASO. Anatomic LVOTO was defined as an echocardiographic peak LVOT gradient exceeding 20 mm Hg associated with an anatomic narrowing. Forty-three patients had a ventricular septal defect. Median follow-up was 7.9 ± 6.5 years (maximum, 25 years). Univariate and multivariate risk analyses for late LVOTO, aortic regurgitation, LVOT reintervention, and death were performed. The early mortality rate was 11% (n = 6); 2 deaths were LVOTO-related. At the latest follow-up, 3 patients (5%) had a LVOTO, 7 (13%) had moderate aortic regurgitation, and 4 (7%) had LVOT reoperation. Actuarial freedom from LVOT reoperation was 90% ± 5% at 10 and 15 years. The mean LVOT peak gradient was 3 ± 9 mm Hg at the latest follow-up. A preoperative pulmonary valve z-score of less than -1.7 (odds ratio, 19; p = 0.02) and an atrioventricular valve-related LVOTO (odds ratio, 15; p = 0.02) are independent predictors of recurrent LVOTO. A preoperative pulmonary valve z-score of less than -1.8 is an independent predictor of LVOT reoperation (odds ratio, 17; p = 0.03). The LVOT gradient per se and the presence of ventricular septal defect or a bicuspid valve do not influence outcomes. Long-term outcomes of ASO for patients with TGA and anatomic LVOTO are satisfactory in selected patients. A lower preoperative pulmonary valve z-score and complex multilevel atrioventricular valve-related LVOTO are independent predictors of recurrent LVOTO and LVOT reoperation. TGA/LVOTO patients with pulmonary valve z-score exceeding -1.8 and resectable valvular or subvalvular LVOTO, or both, should be candidates for ASO, regardless of the severity of the LVOT peak gradient.

Labour analgesia and anaesthetic management of a primigravida with uncorrected Pentology of Fallot

Tetraology of Fallot is the most common congenital heart disease causing intracardiac right-left shunts. It is characterized by presence of ventricular septal defect, aortic overriding, pulmonary artery outflow obstruction and right ventricular hypertrophy. When these features are associated with atrial septal defect, it is often referred to as Pentology of Fallot (POF). If the lesions remain uncorrected, they can cause significant morbidity and mortality to the patient. Pregnancy and labour in such a patient present with significant haemodynamic changes, which can be challenging to the anaesthesiologist. Our patient with POF was managed with labour analgesia and subsequently epidural anaesthesia for drainage of vulval haematoma with successful outcome.

Right Ventricular Function Suffers From Reperfusion Delay: Tissue Doppler Study

In this study, impact of reperfusion delay on adverse cardiac events and right ventricular (RV) function in patients with acute right ventricular myocardial infarction (RVMI) was assessed. In 70 patients with RVMI, RV function was assessed by M-mode tricuspid annular plane systolic excursion (TAPSE) and by pulsed wave tissue Doppler echocardiography (TDE). Right ventricular early (E'(T)) and late diastolic (A'(T)), peak systolic tricuspid annular velocity, (S'(T)) and right ventricular myocardial performance index (RVMPI) were measured. Patients were divided into 2 groups according to the time between the onset of symptoms and percutaneous transluminal coronary angioplasty (PTCA)-group 1 (n = 25), < or =3 hours and group 2 (n = 45), > 3 hours. During 30-day follow-up, we assessed adverse cardiac events like the following: death, cardiogenic shock, need for intra-aortic counterpulsation, temporary transvenous pacing support (PCM), presence of ventricular septal defect (VSD), cardiac tamponade, or free wall rupture. Group 1 had significantly higher values of E'(T), S'(T), and TAPSE than group 2. RVMPI was significantly elevated in group 2. A total of 13 (18.2%) patients had a cardiac event, 5 patients (7.1%) died. We observed an increase of cardiac events in patients with reperfusion delayed more than 3 hours (P = .056). TAPSE-derived and TDE-derived right ventricular systolic and early diastolic velocities were less affected in patients with shorter time from pain onset to opening of the occluded right coronary artery (RCA). Presented initial data show that the tissue Doppler technique may be useful for identification of patients with RV infarction with higher rate of adverse cardiac events including death.

Ventricular septal defect complicating acute myocardial infarction—still an unsolved problem in the invasive treatment era

Post-acute myocardial infarction (AMI) ventricular septal defect (VSD) is a rare but catastrophic complication. The aim of study was to delineate the incidence and risk factors of VSD in patients after AMI treated with successful primary percutaneous coronary intervention (pPCI). In the years 2004-2006, a total of 1835 patients with AMI underwent successful pPCI in our hospital. Thirteen patients (0.71%) developed VSD after pPCI. Mean time of occurrence of VSD was 24.46 ± 9.32 h. Patients with VSD had longer time from the AMI onset to pPCI vs. patients without VSD (7.77 ± 2.83 vs. 4.49 ± 4.45, P<.001). In the VSD group, most of the patients were nonsmokers, had arterial hypertension, and had no previous history of coronary artery disease (CAD). Neither group differed in administered antiplatelet therapy. According to univariate log-regression analysis, the presence of VSD was strongly associated with age >70 years (OR=4.66; P=.007), female gender (OR=5.73; P=.004), anterior infarction (OR=3.86; P=.04), single-vessel CAD (OR=3.74; P=.03), body mass index (BMI) <25 (OR=2.98; P=.04), and left ventricular wall hypertrophy (OR=3.39; P=.03). Our study demonstrated that the incidence of VSD after AMI appears to have declined in patients treated with pPCI. The pathomechanism of VSD in the invasive treatment era is the consequence of several processes and needs further investigation. Advanced age, female gender, anterior infarction, single-vessel CAD, left ventricular wall hypertrophy, and low BMI are strong risk factors of this complication after AMI, which remain invariable over the years.

Chronic Intermittent Materno-Fetal Hyperoxygenation in Late Gestation May Improve on Hypoplastic Cardiovascular Structures Associated with Cardiac Malformations in Human Fetuses

Hypoplasia of cardiovascular structures is a common finding in fetuses with cardiac malformations. Materno-fetal hyperoxygenation (HO) during late gestation promotes venous return to the fetal heart. This analysis in human fetuses sought to define whether this “loading” effect might improve hypoplastic cardiovascular dimensions. Fifteen late-gestation fetuses presented with varying degrees of hypoplastic cardiovascular structures. In these cases, chronic intermittent materno-fetal HO was administered during periods ranging from 8 to 33 days. Cardiac measurements were taken before and at the end of treatment and translated into Z-scores as well as plotted on normal growth charts. During the treatment period, chronic intermittent materno-fetal HO was associated with improved dimensions of ≥1 hypoplastic cardiovascular structures in most fetuses. However, in some cases, the effect of HO was neutralized or impaired by the presence of ventricular septal defects as well as obstructions to ventricular filling or emptying. Chronic intermittent materno-fetal HO near term may be associated with improvements of hypoplastic cardiovascular dimensions in fetuses with a spectrum of cardiac malformations. This effect may facilitate postnatal treatment and improve prognosis in suitable cases.

Cardiac conotruncal malformations in a family of beagle dogs

To study forms of congenital heart disease present in a family of beagle dogs with a strong prevalence of ventricular septal defect and to document the pathological findings associated with the ventricular septal defects and investigate the mode of transmission. The animals were investigated by physical examination, radiography, electrocardiography and ultrasonography to diagnose the presence of congenital heart disease. Some animals were diagnosed at post-mortem examination and the dead animals underwent post-mortem examination to verify the presence of ventricular septal defect. An analysis of pedigree was undertaken and two of the affected animals were mated to investigate the mode of transmission. Among the 28 dogs evaluated clinically or by post-mortem examination, 14 cases of ventricular septal defect were identified. The post-mortem examination showed some abnormalities of the ventricular outflow region associated with malformation of conotruncal septum development. When two affected dogs were bred, congenital heart disease was present in all of the offspring. The congenital heart disease identified in this beagle family can be classified as conotruncal malformation, and an autosomal recessive mode of inheritance was suggested by pedigree analysis.

Improvement in long-term survival after hospital discharge but not in freedom from reoperation after the change from atrial to arterial switch for transposition of the great arteries

To compare survival, freedom from reoperation, and functional status between atrial switch and arterial switch operations for transposition of the great arteries. Data from 88, 329, and 512 patients who underwent Mustard, Senning, and arterial switch operations between 1974 and 2006 were analyzed. In-hospital mortalities were 8.0% for Mustard, 4.6% for Senning, and 6.4% for arterial switch. Presence of ventricular septal defect (hazard ratio 3.3, P < .001) was the only risk factor for in-hospital mortality in multivariate analysis. Follow-up for Mustard was 22.6 +/- 8.1 years, for Senning was 18.2 +/- 5.7 years, and for arterial switch was 9.5 +/- 5.7 years. Highest survival at 20 years was after arterial switch (96.6% +/- 1.3%), followed by Senning (92.6% +/- 1.5%) and Mustard (82.4% +/- 4.3%). Transposition with ventricular septal defect (hazard ratio 3.1, P < .001), transposition with ventricular septal defect and left ventricular outflow tract obstruction (hazard ratio 3.0, P = .029), and Mustard operation (hazard ratio 2.1, P = .011) emerged as risk factors for late death, with arterial switch a protective factor (hazard ratio 0.3, P = .010). Highest freedom from reoperation at 20 years was after Senning (88.7% +/- 1.9%), followed by arterial switch (75.0% +/- 6.4%) and Mustard (70.6% +/- 5.4%). Presence of complex transposition (hazard ratio 2.1, P < .001), previous palliative operation (hazard ratio 1.8, P = .016), surgery between 1985 and 1995 (hazard ratio 2.6, P = .002), surgery after 1995 (hazard ratio 3.5, P < .001), and Mustard operation (hazard ratio 3.3, P < .001) emerged as risk factors for reoperation. Change from atrial to arterial switch led to improved long-term survival after hospital discharge but not to lower incidence of reoperation. Survival and freedom from reoperation are determined by morphology.

Echocardiographic evaluation of ventricular septal defect haemodynamics

Ventricular septal defect (VSD) is an opening in the interventricular septum. 30-50% of patients with congenital heart disease have VSD. The aim of the study was to determine the dependence of the left ventricular diastolic dimension (LVD), left ventricular systolic dimension (LVS), shortening fraction (SF), left atrium (LA), pulmonary artery truncus (TPA) on the body surface and compare their values among experimental, control and a group of healthy children. Values of maximal systolic gradient pressure (Pvsd) of VSD were compared with children from one experimental and control group. Children were divided into three groups: experimental (32 children with VSD that were to go to surgery), control (20 children with VSD who did not require surgery) and 40 healthy children. Measurements of LVD, LVS, SF, LA, TPA were performed in accordance to recommendations of the American Echocardiographic Association. The value of Pvsd was calculated from the maximal flow velocity (V) in VSD using the following formula: Pvsd = 4 x V2 (mm Hg). For children from the experimental group, the relationship between the body surface and the variability of the LVD was explained with 56.85%, LVS with 66.15%, SF with 4.9%, TPA with 58.92%. For children from the control group, the relationship between the body surface and the variability of LVD was explained with 88.8%, LVS with 72.5%, SF with 0.42%, PA with 58.92%. For healthy children, the relationship between the body surface and the variabilitiy of the LVD was explained with 88.8%, LVS with 88.78%, SF with 5.25% and PA with 84.75%. There was a significant statistical difference between average values of Pvsd in the experimental and control group (p < 0.02). The presence of the large VSD has an influence on the enlargement of LVD, LVS, SF,TPA. The enlargment of the size of the pulmonary artery depends on the presence of VSD and there is a direct variation in the magnitude of the shunt. There is a relationship and significant dependence of the LVS and LVD on the body surface. There is no statistically significant dependence between SF and body surface.